Mandible & maxilla
Benign odontogenic tumors
Cemento-ossifying fibroma / ossifying fibroma
Editor-in-Chief: Debra L. Zynger, M.D.
Last author update: 1 May 2018
Last staff update: 6 September 2023 (update in progress)
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PubMed Search: Ossifying fibroma mandible
Table of Contents
Definition / general | Essential features | Terminology | Epidemiology | Sites | Pathophysiology | Etiology | Clinical features | Diagnosis | Radiology description | Radiology images | Prognostic factors | Case reports | Treatment | Clinical images | Gross description | Gross images | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Negative stains | Molecular / cytogenetics description | Molecular / cytogenetics images | Differential diagnosis | Additional references | Board review style question #1 | Board review style answer #1 | Board review style question #2 | Board review style answer #2Cite this page: Martinez A. Cemento-ossifying fibroma / ossifying fibroma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/mandiblemaxillaossifyingfibroma.html. Accessed October 1st, 2023.
Definition / general
- Benign fibro-osseous neoplasm
- Two general categories:
- Ossifying fibroma (discussed in this topic)
- Juvenile ossifying fibroma
- Usually requires surgical excision
Essential features
- Two general categories:
- Ossifying fibroma (discussed in this topic)
- Juvenile ossifying fibroma
- Rarely, ossifying fibroma is associated with syndromes:
Terminology
- Cemento-ossifying fibroma
- Cementifying fibroma
- Periodontoma
Epidemiology
- Peak incidence third-fourth decade of life (Head Neck Pathol 2014;8:432)
- Female predilection (Head Neck Pathol 2014;8:432)
Sites
- Occurs exclusively in the tooth bearing areas of the mandible and maxilla
- Mandible most common location
- Odontogenic origin favored (El-Naggar: WHO Classification of Head and Neck Tumours, 4th Edition, 2017)
Pathophysiology
- Largely unknown for sporadic ossifying fibroma
- Can rarely show syndromic / inherited association:
- Hyperparathyroidism jaw tumor syndrome (Head Neck Pathol 2014;8:432)
- Rare autosomal dominant cause of familial hyperparathyroidism associated with ossifying fibromas of the maxillofacial bones and increased risk of parathyroid carcinoma
- Putative tumor suppressor gene CDC73 on chromosome 1q31 implicated in the syndrome, with a multitude of inactivating mutations identified
- Loss of function of tumor suppressor gene CDC73 results in premature truncation of parafibromin protein product
- Familial gigantiform cementoma
- Transmitted as an autosomal dominant trait that is fully penetrant but with variable expressivity
- Molecular studies to characterize specific mutations are currently unavailable
- Whether or not genetically related to gnathodiaphysial dysplasia is currently not known
- Gnathodiaphysial dysplasia
- GDD1 gene, also known as TMEM16E or anoctamin 5 (ANO5) is mapped to a locus on chromosome 11p14.3 - 15.1
- High levels of GDD1 protein expression in normal cardiac and skeletal muscle tissue as well as in growth plate chondrocytes and osteoblasts in bone suggests an important role for GDD1 in the development of the musculoskeletal systems
- Hyperparathyroidism jaw tumor syndrome (Head Neck Pathol 2014;8:432)
Etiology
- Neoplastic cells elaborate bone / cementum (Head Neck Pathol 2014;8:432)
- Derived from progenitor cells of the periodontal membrane, capable of differentiation into fibroblasts, osteoblasts and cementoblasts (Head Neck Pathol 2014;8:432)
Clinical features
- Sporadic (most common)
- Painless expansion of buccal or lingual plates of jaw
- Large lesions can expand the inferior mandibular border
- Isolated lesion
- Syndromic or Inherited
- Hyperparathyroidism jaw tumor syndrome
- Similar to ossifying fibroma, however may be multiple
- Hyperparathyroidism
- Risk for parathyroid carcinoma
- Familial gigantiform cementoma
- Multifocal / multiquadrant expansive lesions of the jaws, which can be massive and cause remarkable facial deformity
- No other bones are affected
- Lesions arise during childhood and progressively expand to cause facial deformity during early adult years
- Gnathodiaphysial dysplasia
- Multifocal / multiquadrant expansile cemento-ossifying fibroma lesions of the jaws which can be massive
- Extragnathic skeletal abnormalities variably described as osteopenia, osteogenesis imperfecta, fragile and brittle bone disease that lead to multiple fractures, bowing and cortical thickening of the tubular bone
- Hyperparathyroidism jaw tumor syndrome
Diagnosis
- Diagnosis dependent on clinical, radiologic and pathologic correlation
Radiology description
- Expansile
- Early lesions can be radiolucent but progressively more radiopaque over time
Radiology images
Images hosted on other servers:
Fractures in the zygomatic bone and mandibular lesion
Mass filling the right nasal cavity with bony involvement and mild contrast uptake
Prognostic factors
- Slow growing, benign neoplasm
- Uncommon recurrence
Case reports
- 6 year old boy and 19 year old woman with multiple ossifying fibromas of the jaw (Diagn Pathol 2014;9:75)
- 15 year old girl with huge ossifying fibroma of maxilla (Natl J Maxillofac Surg 2015;6:241)
- 30 year old woman with cemento-ossifying fibroma of the mandible (Saudi Med J 2017;38:541)
- 2 patients with CDC73 gene mutations in sporadic ossifying fibroma of the jaw (Diagn Pathol 2016;11:91)
- 13 subjects with ossifying fibroma of the maxilla and sinonasal tract (Allergy Rhinol (Providence) 2017;8:32)
Treatment
- Conservative excision unless massive, which would require en bloc surgical removal (Head Neck Pathol 2014;8:432, Oral Dis 2017;23:440)
Clinical images
Images hosted on other servers:
Large maxillary tumor
Right mandible tumor
Gross description
- Rubbery tan fragments with possible mineralization, gritty
Gross images
Images hosted on other servers:
Enucleated tumor
Large maxillary tumor
Microscopic (histologic) description
- Well defined and may be encapsulated
- Usually has hypercellularity of stroma with mineralized matrix
- Stromal fibroblastic cells have hyperchromatic nuclei; mitoses are not easily found
- Matrix: may see woven and lamellar bone deposits or cementum-like calcifications distributed throughout the lesion
- Scant osteoclasts
- Calcified structures are composed of variable amounts of osteoid or bone and lobulated basophilic masses of cementum-like tissue resembling the cementicles that are normally found in the periodontal membrane; these structures may coalesce and form curvilinear trabeculae which may be acellular
- Ratio of bone to cementum-like tissue varies; one or the other type of the calcified tissue may dominate
- Sharpey's fibers may be seen to projecting radially from spheroidal bone deposits but are not essential for diagnosis (Head Neck Pathol 2008;2:177)
Microscopic (histologic) images
Contributed by Kelly Magliocca D.D.S., M.P.H.
Ossifying fibroma
Mandibular ossifying fibroma
Positive stains
- Variable EMA, variable nuclear beta-catenin
Molecular / cytogenetics description
- No GNAS mutation identified in ossifying fibroma
- Hyperparathyroidism jaw tumor syndrome
- Putative tumor suppressor gene CDC73 on chromosome 1q31 implicated in the syndrome, with a multitude of inactivating mutations identified
- Loss of function of tumor suppressor gene CDC73 results in premature truncation of parafibromin protein product
- Gnathodiaphysial dysplasia
- GDD1 gene, also known as TMEM16E or anoctamin 5 (ANO5) is mapped to a locus on chromosome 11p14.3 - 15.1
Molecular / cytogenetics images
Images hosted on other servers:
CDC73 mutations in sporadic tumors
Differential diagnosis
- Oral cavity/gingival lesion:
- Peripheral ossifying fibroma:
- Most commonly small, although the origin of massive lesions can be difficult to identify without the use cross sectional imaging (Ann Maxillofac Surg 2016;6:300)
- Punctate mineralized spherules or frank ossification in stroma
- Reactive lesion
- Ulcerated or intact squamous mucosa
- Underlying stroma with plump fibroblasts
- Peripheral ossifying fibroma:
- Intraosseous lesion may be problematic to distinguish between entities below when sample consists of small and fragmented biopsy tissues: histologic distinction from ossifying fibroma is not always reliable, emphasizing the importance of radiographic correlation
- Ameloblastoma with desmoplastic features / stroma with associated reactive bone
- Benign proliferative fibro-osseous lesion of temporal bone (Bullough's bump)
- Cemento-osseous dysplasia
- Chronic osteomyelitis: ossification, fibrosis, depletion of giant cells and proliferation of mononuclear cells (Hum Pathol 2017;63:89)
- Desmoplastic fibroma
- Fibrous dysplasia
- Intraosseous meningioma: rare
- Juvenile ossifying fibroma
- Low grade spindle cell sarcoma, NOS with associated reactive bone
- Osteoblastoma
- Reactive bone ("proliferative periostitis")
- Sclerosing odontogenic carcinoma with associated reactive bone or associated fibro-osseous proliferation
- Well differentiated osteosarcoma
- Infiltrative, relatively hypocellular and consists of monomorphic, minimally atypical spindle cells that are usually arranged in fascicles
- Tumor cells are intimately related to the surfaces of elongated trabeculae of neoplastic woven bone that lack osteoblastic rimming (Am J Surg Pathol 2010;34:1647)
Additional references
Board review style question #1
- Which of the following conditions is not typically associated with ossifying fibroma?
- Basal cell nevus syndrome
- Gigantiform familial cementoma
- Gnathodiaphysial dysplasia
- Hyperparathyroidism jaw tumor syndrome
Board review style answer #1
A. Basal cell nevus syndrome is not associated with gnathic ossifying fibroma. It is associated with odontogenic keratocysts of the jaw.
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Reference: Cemento-ossifying fibroma / ossifying fibroma
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Reference: Cemento-ossifying fibroma / ossifying fibroma
Board review style question #2
- What are the collagenous appearing fibers radially projecting from the mineralized deposits?
- Amianthoid fibers
- Osteoblasts
- Rosenthal fibers
- Sharpey's fibers
Board review style answer #2
